The authors present a pedigree where in three generations in male members deep excavations of the optic discs along with Best´s disease of the centres in a variable form were recorded. The heredity is autosomal dominant. Retinal changes conditioned by defects of the pigmented epithelium reduce vision to a maximum of 6/60. The anomaly is associated with a pathological EOG record and normal ERG response. The obser- vation is a contribution to discussions in the literature about the hereditary character of excavations of the optic nerve.

        Key words: deep excavations of the optic disc, Best´s dystrophy, heredity